Words similar to fibrillin
Example sentences for: fibrillin
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Each has a signal sequence (55 amino acids and 49, respectively), a second domain containing an EGF-like repeat, a proline/glycine rich region, a fibrillin motif [ 17 ] , an EGF calcium-binding repeat, and a TGF-β repeat (390 and 389 amino acids, respectively), a third domain containing a proline/glycine rich region (113 and 111 amino acids, respectively), a fourth domain containing numerous calcium-binding type II EGF-like modules (D/N)(I/V)(D/N)(E/D)C 1 (678 and 677 amino acids, respectively) and a fifth carboxyl-terminal domain (5 and 21 amino acids, respectively) (human LTBP-3 information was obtained from the GenBank database accession no.
The deposition of newly synthesized fibrillin into the matrix was greatly reduced (13 % of normal), however, suggesting that the internally deleted mutant fibrillin-1 molecules are secreted and exert a dominant-negative effect on extracellular microfibril assembly.
It is well established that EGF-like modules are important structural features found in many extracellular proteins and that these modules enable numerous protein :protein interactions; the binding of EGF-like modules in laminin to nidogen [ 19 ] and the interaction of EGF-like modules within fibrillin monomers [ 20 21 ] . Calcium may be required for some of these protein interactions.
3. In addition, we found that certain cysteine substitutions in EGF-like domains and located immediately N-terminal of LTBP-like domains, cause protein instability and greatly reduced matrix deposition [ 9 ] . Taken together, our data support a model in which misfolding or partial deletion of LTBP-like domains results in proteolytic cleavage of the mutant fibrillin molecules.
In this patient's fibroblasts, fibrillin synthesis was 41% of control and deposition was reduced to 5%, while the mutant mRNA level was normal as shown here in Fig.
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